Hereditary hemorrhagic telangiectasia (OslerWeberRendu syndrome) - Part II. Pharmacological therapy and international guidelines for the therapy 2020

Vnitr Lek. 2021 Winter;67(7):419-424.

Abstract

Hereditary hemorrhagic telangiectasia also known as Osler-Weber-Rendu syndrome, is an disorder that causes abnormal blood vessel formation with bleeding. Inhibition of angiogenesis amelioretes bleeding complication. Anti-angiogenic agents such as bevacizumab, aflibercept, thalidomid, lenadomid and other new anti-angiogenic thyrosinkinase inhibitors, as well as sirolimus and takrolimus have emerged as a promising systemic or local therapy in reducing bleeding complications but are not curative. Other pharmacological agents include iron supplementation, antifibrinolytics and hormonal treatment. This review concentrates on new anti-agioproliferative drugs with effect in HHT- discusses the new biology of HHT, management issues that face the practising hematologist, and considerations of future directions in HHT treatment.

Keywords: aflibercept; bevacizumab; hereditary hemorrhagic telangiectasia; lenadomid anti‑angiogenic thyrosinkinase inhibitors; lenalidomid; sirolimus; tacrolimus; thalidomid; thalidomide.

Publication types

  • Review

MeSH terms

  • Angiogenesis Inhibitors / therapeutic use
  • Bevacizumab / therapeutic use
  • Hemorrhage / complications
  • Humans
  • Syndrome
  • Telangiectasia, Hereditary Hemorrhagic* / complications
  • Telangiectasia, Hereditary Hemorrhagic* / drug therapy

Substances

  • Angiogenesis Inhibitors
  • Bevacizumab